Abstract: Desmoplastic small round cell tumors(DSRCTs) are a rare malignancy found in male adolescents that initially occur mostly in the abdominal cavity.Diagnosis is based on the histologic analysis of biopsies, which typically show small round blue cells in nests separated by abundant desmoplastic stroma.DSRCTs are associated with a unique chromosomal translocation t(11:22)(p 13;q 12) that involves the Ewing's sarcoma(EWS) gene and the Wilms' tumor(WT1) gene.Reverse transcriptase-polymerase chain reaction can be used to detect the fusion gene in fresh or paraffin-embedded tissues, which confirms the diagnosis.The prognosis is particularly poor.The median survival ranges from 17 to 25 months.Management of DSRCT remains challenging despite the use of aggressive therapies such as polychemotherapy, debulking surgery, and whole abdominal radiation.Several methods for improving patient survival are being evaluated, such as the addition of chemotherapy and targeted therapies to normal neoadjuvant protocols, complete surgical resection with hyperthermic intraperitoneal chemotherapy, postoperative intensity-modulated radiation therapy, and yttrium-90 microsphere liver embolization for treating hepatic metastases.